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C9orf72 poly(PR) mediated neurodegeneration is associated with nucleolar stress

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The ALS/FTD-linked intronic hexanucleotide repeat expansion in the C9orf72 gene is translated into dipeptide repeat proteins, among which poly-proline-arginine (PR) displays the most aggressive neurotoxicity in-vitro and in-vivo. PR partitions to the nucleus when expressed in neurons and other cell...

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Detalles Bibliográficos
Autores principales:	Cicardi, ME, Hallgren, JH, Mawrie, D, Krishnamurthy, K, Markandaiah, SS, Nelson, AT, Kankate, V, Anderson, EN, Pasinelli, P, Pandey, UB, Eischen, CM, Trotti, D
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Cold Spring Harbor Laboratory 2023
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9949130/ https://www.ncbi.nlm.nih.gov/pubmed/36824930 http://dx.doi.org/10.1101/2023.02.16.528809

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